Guillain Barre Syndrome

Journal Reading Fathia Rachmatina 030.08.099

Definisi
Guillain Barre syndrome ( GBS ) adalah suatu kelainan sistem kekebalan tubuh manusia yang menyerang bagian dari susunan saraf tepi dirinya sendiri dengan karekterisasi berupa kelemahan atau arefleksia dari saraf motorik yang sifatnya progresif. Kelainan ini kadang kadang juga menyerang saraf sensoris, otonom, maupun susunan saraf pusat.

Epidemiologi
The reported incidence of GBS in Western countries ranges from 0.89 to 1.89 cases per 100.000 person-years The incidence increases gradually with age, but the disease may occur at any age. Men and women are affected equally

Etiologi
Infeksi GBS sering sekali berhubungan dengan infeksi akut non spesifik. Insidensi kasus GBS yang berkaitan dengan infeksi ini sekitar antara 56% - 80%, yaitu 1 sampai 4 minggu sebelum gejala neurologi timbul seperti infeksi saluran pernafasan atas atau infeksi gastrointestinal.

 Patogen yang tersering ditemukan adalah Campylobacter jejuni, cytomegalo virus (CMV),Mycoplasmapneumonia, Epstein-Barr virus, dan virus inuenza.

Gejala Klinis
GBS ditandai dengan timbulnya suatu kelumpuhan akut yang disertai hilangnya refleks-refleks tendon dan didahului parestesi dua atau tiga minggu setelah mengalami demam disertai disosiasi sitoalbumin pada likuor dan gangguan sensorik dan motorik perifer.

Variants
Classical GBS Recurrent GBS Miller-Fisher syndrome is characterized by gait ataxia, areflexia, and ophthalmoparesis. Acute sensory polyneuritis Acute panautonomic neuropathy Acute axonal variant of GBS

Miller Fisher Syndrome

The Miller Fisher syndrome appears to be more common among patients with the GuillainBarr syndrome who live in eastern Asia than among those who live in other parts of the world

Most patients with the Miller Fisher syndrome have evidence of infection 1 to 3 weeks before the development of ophthalmoplegia or ataxia; in one study, 20% of patients had C. jejuni infection and 8% had Haemophilus influenzae infection.The presence of distal paresthesia is associated with the Miller Fisher syndrome.

Diagnosis:
Albuminocytologic dissociation: elevated CSF protein w/ normal WBC (80-90% pts) Electromyography (EMG) helps confirm diagnosis = prolonged or absent F waves

Patofisiologi

Terapi
Monitoring cardiac and pulmonary dysfunction Prevention of pulmonary embolism Immunotherapy

Immunotherapy
Plasma exchange was the first treatment that was found to be effective in hastening recovery in patients with the Guillain Barr syndrome, and it appeared to be most effective when it was started within the first 2 weeks after disease onset in patients who were unable to walk.

An electrophysiological examination is not always required for the initiation of immunotherapy. Plasma exchange nonspecifically removes antibodies and complement and appears to be associated with reduced nerve damage and faster clinical improvement, as compared with supportive therapy alone

Prognosis
Pada umumnya penderita mempunyai prognosa yang baik tetapi pada sebagian kecil penderita dapat meninggal atau mempunyai gejala sisa. 95% terjadi penyembuhan tanpa gejala sisa dalam waktu 3 bulan bila dengan keadaan antara lain: pada pemeriksaan NCV-EMG relatif normal mendapat terapi plasmaparesis dalam 4 minggu mulai saat onset progresifitas penyakit lambat dan pendek pada penderita berusia 30-60 tahun